Dystonia and Chorea in Acquired Systemic Disorders
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Cp-Research-News-2014-06-23
Monday 23 June 2014 Cerebral Palsy Alliance is delighted to bring you this free weekly bulletin of the latest published research into cerebral palsy. Our organisation is committed to supporting cerebral palsy research worldwide - through information, education, collaboration and funding. Find out more at www.cpresearch.org.au Professor Nadia Badawi Macquarie Group Foundation Chair of Cerebral Palsy PO Box 560, Darlinghurst, New South Wales 2010 Australia Subscribe at www.cpresearch.org/subscribe/researchnews Unsubscribe at www.cpresearch.org/unsubscribe Interventions and Management 1. Iran J Child Neurol. 2014 Spring;8(2):45-52. Associations between Manual Abilities, Gross Motor Function, Epilepsy, and Mental Capacity in Children with Cerebral Palsy. Gajewska E, Sobieska M, Samborski W. OBJECTIVE: This study aimed to evaluate gross motor function and hand function in children with cerebral palsy to explore their association with epilepsy and mental capacity. MATERIAL & METHODS: The research investigating the association between gross and fine motor function and the presence of epilepsy and/or mental impairment was conducted on a group of 83 children (45 girls, 38 boys). Among them, 41 were diagnosed with quadriplegia, 14 hemiplegia, 18 diplegia, 7 mixed form, and 3 athetosis. A neurologist assessed each child in terms of possible epilepsy and confirmed diagnosis in 35 children. A psychologist assessed the mental level (according to Wechsler) and found 13 children within intellectual norm, 3 children with mild mental impairment, 18 with moderate, 27 with severe, and 22 with profound. Children were then classified based on Gross Motor Function Classification System and Manual Ability Classification Scale. RESULTS: The gross motor function and manual performance were analysed in relation to mental impairment and the presence of epilepsy. -
Oral Contraceptive Induced Chorea: Another Condition Associated with Anti-Basal Ganglia Antibodies M Miranda, F Cardoso, G Giovannoni, a Church
327 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.2003.019851 on 23 January 2004. Downloaded from SHORT REPORT Oral contraceptive induced chorea: another condition associated with anti-basal ganglia antibodies M Miranda, F Cardoso, G Giovannoni, A Church ............................................................................................................................... J Neurol Neurosurg Psychiatry 2004;75:327–328 anti-DNAase B titre of 120 IU/ml was normal (normal Use of oral contraceptives is a recognised but infrequent ,340 IU/ml). Throat cultures did not detect streptococcus. cause of chorea. This type of chorea has usually been Her full blood count, erythrocyte sedimentation rate, C- considered a reactivation of Sydenham’s chorea by an reactive protein, thyroid function, plasma amino acids, unknown mechanism. A patient developed a chorea ceruloplasmin, antinuclear antibodies, and antiphospholipid triggered by the use of oral contraceptives with no definite antibodies were either normal or negative. Magnetic reson- evidence of previous Sydenham’s chorea or recent streptoc- ance imaging of the brain was normal. Echocardiogram cocal infections. However, the patient had positive anti-basal showed no alterations. Anti-basal ganglia antibodies were ganglia antibodies, which supports an immunological basis positive by Western immunoblotting and revealed reactivity for the pathophysiology of this chorea. against antigens of 40 and 45 kDa in size. The antibody assay method has been described previously.4 The patient’s serum was also screened against a liver antigen preparation to exclude non-specific binding as a result of antinuclear factors or other auto-antibodies. These specific 40 and 45 kDa bands se of oral contraceptives is a well known but appear to be relatively specific for the group of disorders 12 uncommon cause of chorea . -
Acute and Chronic Chorea in Childhood Donald L
Acute and Chronic Chorea in Childhood Donald L. Gilbert, MD, MS This review discusses diagnostic evaluation and management of chorea in childhood. Chorea is an involuntary, hyperkinetic movement disorder characterized by continuous, jerky, or flowing movement fragments, with irregular timing and direction. It tends to be enhanced by voluntary actions and generally causes interference with fine motor function. The diagnostic evaluation begins with accurate classification of the movement disorder followed by consideration of the time course. Most previously healthy children presenting with acute/subacute chorea have an autoimmune etiology. Chronic chorea usually occurs as part of encephalopathies or diseases causing more global neurologic symptoms. We review the management of acute/subacute and chronic choreas, with special emphasis on Sydenham chorea and benign hereditary chorea. Semin Pediatr Neurol 16:71–76 © 2009 Published by Elsevier Inc. horea is a nonpatterned, involuntary, hyperkinetic genetic chorea, will be emphasized. Paroxysmal movement Cmovement disorder. It is continuous, variable in speed, disorders involving chorea will not be discussed but are re- unpredictable in timing and direction, and flowing or jerky in viewed elsewhere.4 As the phenomenology of chorea over- appearance.1 Chorea may be accompanied by athetosis or laps in acute and chronic choreas, most features of the neu- ballism. Athetosis is also continuous but the rate is slower. rologic examination will be discussed under acute chorea. Athetosis often accompanies dystonia or occurs in symptom- atic chorea and may be referred to as choreoathetosis. Ballism designates larger amplitude, flinging, proximally generated Acute Chorea movements. It rarely occurs in isolation in children but can accompany chorea. -
Wilson's Disease
Reprinted with permission from Thieme Medical Publishers (Semin Neurol. 2007 April;27(2):123-132) Homepage at www.thieme.com Wilson’s Disease Ronald F. Pfeiffer, M.D.1 ABSTRACT Wilson’s disease is an autosomal-recessive disorder caused by mutation in the ATP7B gene, with resultant impairment of biliary excretion of copper. Subsequent copper accumulation, first in the liver but ultimately in the brain and other tissues, produces protean clinical manifestations that may include hepatic, neurological, psychiatric, oph- thalmological, and other derangements. Genetic testing is impractical because of the multitude of mutations that have been identified, so accurate diagnosis relies on judicious use of a battery of laboratory and other diagnostic tests. Lifelong palliative treatment with a growing stable of medications, or with liver transplantation if needed, can successfully ameliorate or prevent the progressive deterioration and eventual death that would otherwise inevitably ensue. This article discusses the epidemiology, genetics, pathophysi- ology, clinical features, diagnostic testing, and treatment of Wilson’s disease. KEYWORDS: Ceruloplasmin, copper, Wilson’s disease, penicillamine, zinc Although he was not the first to recognize the EPIDEMIOLOGY disease process,1 in a doctoral thesis of more than 200 Wilson’s disease is a rare autosomal-recessive disorder. A pages published in Brain in 1912, S. A. Kinnier Wilson prevalence rate of 30 cases per million (or one per masterfully provided the first detailed, coherent descrip- 30,000) and a birth incidence rate of one per 30,000 to 12–15 tion of both the clinical and pathological details of the 40,000 are often quoted. It has been estimated that entity that now bears his name.2 Many other individuals there are 600 cases of Wilson’s disease in the United 14 have embellished and expanded our understanding of States and that 1% of the population are carriers. -
Abnormal Eye Movements in Three Types of Chorea
DOI: 10.1590/0004-282X20160109 VIEW AND REVIEW Abnormal eye movements in three types of chorea Anormalidades dos movimentos oculares em três tipos de coreia Tiago Attoni1, Rogério Beato2, Serge Pinto3, Francisco Cardoso2 ABSTRACT Chorea is an abnormal movement characterized by a continuous flow of random muscle contractions. This phenomenon has several causes, such as infectious and degenerative processes. Chorea results from basal ganglia dysfunction. As the control of the eye movements is related to the basal ganglia, it is expected, therefore, that is altered in diseases related to chorea. Sydenham’s chorea, Huntington’s disease and neuroacanthocytosis are described in this review as basal ganglia illnesses that can present with abnormal eye movements. Ocular changes resulting from dysfunction of the basal ganglia are apparent in saccade tasks, slow pursuit, setting a target and anti-saccade tasks. The purpose of this article is to review the main characteristics of eye motion in these three forms of chorea. Keywords: chorea; eye movements; Huntington disease; neuroacanthocytosis. RESUMO Coreia é um movimento anormal caracterizado pelo fluxo contínuo de contrações musculares ao acaso. Este fenômeno possui variadas causas, como processos infecciosos e degenerativos. A coreia resulta de disfunção dos núcleos da base, os quais estão envolvidos no controle da motricidade ocular. É esperado, então, que esta esteja alterada em doenças com coreia. A coreia de Sydenham, a doença de Huntington e a neuroacantocitose são apresentadas como modelos que têm por característica este distúrbio do movimento, por ocorrência de processos que acometem os núcleos da base. As alterações oculares decorrentes de disfunção dos núcleos da base se manifestam em tarefas de sacadas, perseguição lenta, fixação de um alvo e em tarefas de antissacadas. -
PPCO Twist System
PEER REVIEWED The NEUROLOGICNEUROLOGIC EXAMINATIONEXAMINATION in Companion Animals In the January/February issue of Part 2: Interpreting Today’s Veterinary Practice, Part 1 of this article discussed performing Abnormal Findings a neurologic examination; Part 2 will address interpretation of Helena Rylander, DVM, Diplomate ACVIM (Neurology) abnormal findings. complete neurologic examination should be THE BRAIN done in all animals presenting with suspected Lesions in the brain can be localized to the: Aneurologic disease. Abnormalities found during t Cerebrum and thalamus (ie, prosencephalon) the neurologic examination can reflect the location of t Brainstem the lesion, but not the cause, requiring further tests, t Cerebellum. such as blood analysis, electrodiagnostic tests, and In order to localize the lesion to a specific part of advanced imaging, to determine a diagnosis. the brain, an understanding of the anatomy and func- The neurologic examination evaluates different parts tion of the brain is necessary (see Brain Anatomy & of the nervous system; the findings from the examina- Related Functions). tion help localize the lesion to the: t Brain Ataxia t Spinal cord A patient with ataxia may have a lesion in the proprio- t Peripheral nervous system ceptive pathways (peripheral nerves, spinal cord, or t Cauda equina. cerebrum), vestibular system, or cerebellum. Ataxia A fundic examination is recommended, especially in can be described as an uncoordinated gait, with cross- patients with brain disorders. Repeat neurologic exami- ing of the limbs and, sometimes, listing or falling to 1 nations are helpful to discover subtle abnormalities and or both sides. Ataxia can be further characterized as: assess progression of disease. -
The Dystonias
LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES SUBJECT REVIEW The Dystonias Edith G. McGeer and Patrick L. McGeer Can. J. Neurol. Sci. 1988; 15: 447-483 Contents This review is intended for both the practitioner and the sci entist. Its purpose is to summarize current knowledge regarding the various forms of dystonia, as well as the pathology known Introduction to produce the syndrome in specialized circumstances. Histopathological and brain imaging studies The low incidence of the disorder, its prolonged course, and the difficulty of accurate diagnosis has precluded the type of Sleep and other physiological studies systematic investigation that is possible with many other disor Chemical pathology ders. Yet such systematic investigation is essential if the myster Brain studies ies surrounding dystonia are to be unravelled and methods of CSF studies treatment improved. Blood studies Dystonia has been defined by the Scientific Advisory Board Urine studies of the Dystonia Medical Research Foundation as a syndrome of Fibroblast studies sustained muscle contraction, frequently causing twisting and Miscellaneous repetitive movements, or abnormal posture. It is a clinical term Therapy and not a disease description. It refers to all anatomical forms, whether they involve generalized musculature or only focal Iatrogenic dystonia groups. Although dystonia appears as part of the syndrome in a Possible animal models of dystonia number of disease states, it is idiopathic dystonia, where inheri Summary tance is a major factor, that has aroused the greatest medical interest. This review emphasizes recent literature and those aspects which may contribute to an understanding of the under 1. INTRODUCTION lying mechanisms of dystonic movement. -
The Rostrocaudal Gradient for Somatosensory Perception in The
692 J Neurol Neurosurg Psychiatry 2000;69:692–709 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.5.692 on 1 November 2000. Downloaded from A 49 year old right handed man suddenly shape, and texture weighing 50, 60, 70, 80, developed dysaesthesia in the right hand. 90, and 100 g. For texture perception, we LETTERS TO This recovered gradually, but 1 month later prepared six wooden plates of an identical he still had an impaired tactile recognition for size and shape, on which one of six diVerent THE EDITOR objects. His voluntary movements were skill- textures (sandpaper, felt, wood, wool, fine ful. Deep tendon reflex was slightly exagger- grain, synthetic rubber) were mounted. The ated in his right arm. Babinski’s sign was patient palpated one texture by either hand absent. His language was normal. Brain MRI with his eyes closed. Then he was asked to on the 35th day after the onset showed a select tactually a correct one among the six The rostrocaudal gradient for laminar necrosis on the caudal edge of the textures. For shape perception (three somatosensory perception in the human lateral portion of the left postcentral gyrus dimensional figures) the patient palpated one postcentral gyrus (figure). of the five wooden objects (cylinder, cube, Somaesthetic assessment was done during sphere, prism, and cone) with his eyes closed. Anatomical organisation of the primate post- the 21–28th days of the illness. Then he was asked to explain the shape ver- central gyrus has been described in terms of Elementary somatosensory functions were bally. -
History-Of-Movement-Disorders.Pdf
Comp. by: NJayamalathiProof0000876237 Date:20/11/08 Time:10:08:14 Stage:First Proof File Path://spiina1001z/Womat/Production/PRODENV/0000000001/0000011393/0000000016/ 0000876237.3D Proof by: QC by: ProjectAcronym:BS:FINGER Volume:02133 Handbook of Clinical Neurology, Vol. 95 (3rd series) History of Neurology S. Finger, F. Boller, K.L. Tyler, Editors # 2009 Elsevier B.V. All rights reserved Chapter 33 The history of movement disorders DOUGLAS J. LANSKA* Veterans Affairs Medical Center, Tomah, WI, USA, and University of Wisconsin School of Medicine and Public Health, Madison, WI, USA THE BASAL GANGLIA AND DISORDERS Eduard Hitzig (1838–1907) on the cerebral cortex of dogs OF MOVEMENT (Fritsch and Hitzig, 1870/1960), British physiologist Distinction between cortex, white matter, David Ferrier’s (1843–1928) stimulation and ablation and subcortical nuclei experiments on rabbits, cats, dogs and primates begun in 1873 (Ferrier, 1876), and Jackson’s careful clinical The distinction between cortex, white matter, and sub- and clinical-pathologic studies in people (late 1860s cortical nuclei was appreciated by Andreas Vesalius and early 1870s) that the role of the motor cortex was (1514–1564) and Francisco Piccolomini (1520–1604) in appreciated, so that by 1876 Jackson could consider the the 16th century (Vesalius, 1542; Piccolomini, 1630; “motor centers in Hitzig and Ferrier’s region ...higher Goetz et al., 2001a), and a century later British physician in degree of evolution that the corpus striatum” Thomas Willis (1621–1675) implicated the corpus -
Post Stroke Focal Aware Seizures Presenting As Delayed Onset Choreoathetosis
Lehigh Valley Health Network LVHN Scholarly Works Department of Medicine Post Stroke Focal Aware Seizures Presenting as Delayed Onset Choreoathetosis Artish Patel USF MCOM - LVHN Campus, [email protected] Patrick Davis USF MCOM - LVHN Campus, [email protected] Beth Stepanczuk MD Lehigh Valley Health Network, [email protected] Follow this and additional works at: https://scholarlyworks.lvhn.org/medicine Part of the Medicine and Health Sciences Commons Published In/Presented At Patel, A., Davis, P., & Stepanczuk, B. (2021, February 9-13). Post Stroke Focal Aware Seizures Presenting as Delayed Onset Choreoathetosis. [Poster presentation]. Association of Academic Physiatrists Annual Meeting, Virtual. This Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by an authorized administrator. For more information, please contact [email protected]. Post Stroke Focal Aware Seizures Presenting as Delayed Onset Choreoathetosis Artish Patel, MD, BS, Patrick Davis, MD, BS, and Beth Stepanczuk, MD Lehigh Valley Health Network; Allentown, Pa. Setting Case Description Discussion Outpatient follow-up office She initially presented with acute onset confusion, nausea, This patient offered a rare case of focal aware seizures vomiting, and aphasia. Imaging revealed acute left parietal presenting as delayed onset choreoathetosis of the right Patient temporal intraparenchymal hemorrhage with surrounding upper extremity secondary to acute left parietal temporal 39-year-old female with history of migraines and recent left parietal edema and restricted diffusion consistent with infarction intraparenchymal hemorrhage. It has been documented that temporal intraparenchymal hemorrhage presenting at three- secondary to superior sagittal sinus thrombosis. -
Paraneoplastic Neurological and Muscular Syndromes
Paraneoplastic neurological and muscular syndromes Short compendium Version 4.5, April 2016 By Finn E. Somnier, M.D., D.Sc. (Med.), copyright ® Department of Autoimmunology and Biomarkers, Statens Serum Institut, Copenhagen, Denmark 30/01/2016, Copyright, Finn E. Somnier, MD., D.S. (Med.) Table of contents PARANEOPLASTIC NEUROLOGICAL SYNDROMES .................................................... 4 DEFINITION, SPECIAL FEATURES, IMMUNE MECHANISMS ................................................................ 4 SHORT INTRODUCTION TO THE IMMUNE SYSTEM .................................................. 7 DIAGNOSTIC STRATEGY ..................................................................................................... 12 THERAPEUTIC CONSIDERATIONS .................................................................................. 18 SYNDROMES OF THE CENTRAL NERVOUS SYSTEM ................................................ 22 MORVAN’S FIBRILLARY CHOREA ................................................................................................ 22 PARANEOPLASTIC CEREBELLAR DEGENERATION (PCD) ...................................................... 24 Anti-Hu syndrome .................................................................................................................. 25 Anti-Yo syndrome ................................................................................................................... 26 Anti-CV2 / CRMP5 syndrome ............................................................................................ -
The Clinical Approach to Movement Disorders Wilson F
REVIEWS The clinical approach to movement disorders Wilson F. Abdo, Bart P. C. van de Warrenburg, David J. Burn, Niall P. Quinn and Bastiaan R. Bloem Abstract | Movement disorders are commonly encountered in the clinic. In this Review, aimed at trainees and general neurologists, we provide a practical step-by-step approach to help clinicians in their ‘pattern recognition’ of movement disorders, as part of a process that ultimately leads to the diagnosis. The key to success is establishing the phenomenology of the clinical syndrome, which is determined from the specific combination of the dominant movement disorder, other abnormal movements in patients presenting with a mixed movement disorder, and a set of associated neurological and non-neurological abnormalities. Definition of the clinical syndrome in this manner should, in turn, result in a differential diagnosis. Sometimes, simple pattern recognition will suffice and lead directly to the diagnosis, but often ancillary investigations, guided by the dominant movement disorder, are required. We illustrate this diagnostic process for the most common types of movement disorder, namely, akinetic –rigid syndromes and the various types of hyperkinetic disorders (myoclonus, chorea, tics, dystonia and tremor). Abdo, W. F. et al. Nat. Rev. Neurol. 6, 29–37 (2010); doi:10.1038/nrneurol.2009.196 1 Continuing Medical Education online 85 years. The prevalence of essential tremor—the most common form of tremor—is 4% in people aged over This activity has been planned and implemented in accordance 40 years, increasing to 14% in people over 65 years of with the Essential Areas and policies of the Accreditation Council age.2,3 The prevalence of tics in school-age children and for Continuing Medical Education through the joint sponsorship of 4 MedscapeCME and Nature Publishing Group.